A 27-year-old woman was referred for evaluation of a right-sided intranasal mass. Her medical history was significant for recurring unilateral nasal obstruction, sinus infections, epistaxis, and headaches. She also had a history of hyponatremia and seizures due to SIADH (syndrome of inappropriate antidiuretic hormone). A biopsy was performed after an attempt at surgical excision failed because of severe intraoperative hypertension. The results of immunohistochemical staining of the intranasal specimen were positive for the following components: synaptophysin, chromogranin, CD56, S100 protein, glial fibrillary acid protein, and neurofilament. Further workup for the episode of severe hypertension disclosed elevated 24-hour urinary catecholamine levels and mild tracer uptake in the right nasal region on iobenguane I 131 scan. The patient was then referred to our medical center for subsequent treatment.